Patients with benign chordoma have a survival time of more than 5 years. Benign chordoma is a tumor of congenital origin that occurs in the cranial or sacrococcygeal region and has different clinical manifestations depending on the specific location of the tumor. The prognosis for patients with benign chordoma is often good, with treatment options including surgery, radiation therapy, and targeted drug therapy, especially for patients in the sacrococcygeal region. Some patients with low-grade malignant chordoma are more malignant and are prone to local invasion and distant metastases, resulting in a shorter survival time. For patients with sacrococcygeal chordoma, surgery is the mainstay, and early appropriate treatment is generally recommended.